Fig. 1.

Systemic-intraosseous administration of human DEC (1 × 10⁶ and 5 × 10⁶) cells confirms long-term DEC engraftment and increased dystrophin expression in the heart, diaphragm and gastrocnemius muscle at 180 days after DEC transplant to the mdx/scid mice. Immunofluorescence images of dystrophin expression in the heart (A), diaphragm (C) and gastrocnemius muscle (E) after systemic DEC transplant compared to vehicle and WT controls. White arrows indicate positive fibers, showing differences between the therapy groups and the vehicle control; Magnification 25X, scale bar = 50 μm; for merge: dystrophin (green), human spectrin (red), DAPI nuclei counterstaining (blue). (G) Representative immunofluorescence images of human spectrin (red) expression in the heart, diaphragm and gastrocnemius muscle samples of the mdx/scid hosts injected with human DEC (1 × 10⁶ and 5 × 10⁶), dystrophin (green), nuclei counterstained with DAPI (blue); Magnification 140X, scale bar 20 μm (ZEISS 710 META, Oberkochen, Germany). (B, D, F) Significant increase of dystrophin expression in target organs of: heart (B), diaphragm—dose-dependent effect (D), and gastrocnemius muscle (F) of DEC-injected compared to vehicle injected mdx/scid mice, n = 3/group, 10 ROI/organ/mouse. Dystrophin-positive fibers were counted and normalized to the total number of fibers. All data presented as mean ± SEM. One-way ANOVA with post-hoc Tukey’s test. **p < 0.01, *** p < 0.001